Abstract O.36: Kawasaki Disease and Systemic Juvenile Idiopathic Arthritis - Two Ends of the Same Spectrum
Bibliographic record
Abstract
Background: Clinical similarities between Kawasaki disease (KD) and systemic Juvenile Idiopathic Arthritis (sJIA) are well known. We critically reviewed children with KD and sJIA to identify those with both diagnoses in order to characterize discriminating findings at baseline. Methods: Data from prospectively acquired KD (n=1765) and sJIA (n=112) cohorts were reviewed for common patients (1990-2011). Those with both diagnoses (KD/sJIA n=8) were reviewed for clinical presentation, laboratory investigations, treatment regimens, coronary artery outcome and complications including macrophage activation syndrome, and results were compared to the overall KD cohort. Results: All children with KD/sJIA fulfilled diagnostic criteria for KD and sJIA (ILAR classification). Co-diagnosis was present in 0.45% (8 of 1765) and 7.1% (8 of 112) of those with KD and sJIA, respectively. Time between diagnosis of KD and presumptive diagnosis of sJIA was a median of 24 days (IQR 21-45 days). KD/sJIA patients had bilateral conjunctival injection less frequently, lower hepatic transaminases together with signs of more intense inflammation as expressed a by higher white blood cell count and lower albumin than the KD cohort alone. All KD/sJIA patients had recalcitrant disease consisting of prolonged fever requiring multiple doses of intravenous immunoglobulin and steroids. Coronary artery abnormalities (CAAs) were observed in 5 KD/sJIA patients. Macrophage activation syndrome occurred in one KD/sJIA patient and in 0.9% and 8% of KD patients and sJIA patients respectively. Conclusions: A small portion of our patients with KD developed subsequent sJIA. KD/sJIA patients were characterized by more intense inflammation at initial presentation, a recalcitrant disease course and a high prevalence of CAAs. These patients may provide clues to potentially shared immunopathology. The clinical presentations of MAS, KD and sJIA are quite similar with fever, rash, hepatomegaly, and lymphadenopathy. All 3 entities are syndrome complexes defined by massive immune activation. We propose that the intensity and duration of the immune response may be the key distinguishing features, which dictate which one of these clinical syndromes the affected child presents with.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.003 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.001 |
| Science and technology studies | 0.000 | 0.001 |
| Scholarly communication | 0.001 | 0.001 |
| Open science | 0.000 | 0.001 |
| Research integrity | 0.001 | 0.001 |
| Insufficient payload (model declined to judge) | 0.003 | 0.001 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".